Leiomyosarcoma of the duodenum

Abstract

SUMMARYThree cases of leiomyosarcoma of the duodenum are reported. T h e literature is reviewed, and the clinical, diagnostic, and pathological features of this tumour are described. Wide surgical excision has proved to be the most effective treatment.LEIOMYOSARCOMA of the duodenum is an uncommon malignant tumour and is seldom encountered more than once or twice in the working life of any surgeon.I t receives brief mention in a few standard surgicalWe recently encountered a case in clinical practice and were able to find 2 others in the records of St. Peter's Hospital, Chertsey, from 1952 to 1970 CASE REPORTS Case 1.4vh-s. M. M., a housewife aged 53 years, was admitted to hospital in February, 1969. For a month she had felt faint and giddy and 3 days prior to admission had begun to pass melaena stools. She suffered from rheumatoid arthritis and had been treated with aspirin and prednisone for 9 years.On admission she was found to be anaemic (haemog!obin 5.2 g. per IOO ml.), but had no other abnormal signs.A blood transfusion was given but the melaena continued. A barium meal (Fig. I) showed an unusual 'diverticulum' of the third part of the duodenum, with an irregularly narrowed and possibly neoplastic neck. Atonic double-contrast duodenography (Fig. 2) reinforced the suspicion of malignancy and suggested a mass in the duodenal wall in relation to the ' diverticulum'.As the bleeding continued, laparotomy was performed. A large mobile tumour was found in the third part of the duodenum. No other abnormality was found. The third part of the duodenum containing the tumour was carefully mobilized from the pancreas and adjacent vessels, was resected, and an end-to-end anastomosis was performed. The abdomen was closed with drainage. The patient recovered uneventfully and went home on the tenth postoperative day. textbooks, and the majority of the recorded cases Macroscopic examination of the specimen (Fig. 3) in the literature were neither diagnosed preoperarevealed a tumour, 9 X 5 x 2 cm., in the wall of the tively nor recognized at laparotomy. T h e first case duodenum, the lumen. The was repofled by salis in 1920, and only 28 serosa was not involved, but the mucosa was ulcerated over an area 5 cm. in diameter (Fig. 4). MicroscopicOthers had been recorded before I9.53. Since then examination (Figs. 5, 6) showed a cellular, spindle-celled the lesion has been more frequently described. tumour arising in the smooth muscle of the duodenal wall; occasional mitoses were seen. The diagnosis was leiomyosarcoma (Dr. A. C. Cornsell).The patient is alive and in good health I year after her operation.

Duodenal Leiomyosarcoma

“…It is therefore not surprising that only 15–28% of small bowel leiomyosarcomas arise from the duodenum and that barely 10% of all duodenal malignancies are of this type [4, 9, 10]. The distribution of these lesions within the duodenum in our series agrees with that reported in the literature, approximately half being reported in D2, one third in D3 and less than 10% in either D4 or D1 [1, 4, 11]. …”

“…Their growth pattern is typically exophytic (63%), which explains the rarity of biliary or duodenal obstruction; none of our patients had vomiting or jaundice. Endophytic (23%) and dumb-bell types (14%) have also been described [4]. The common cystic appearance on CT is explained by central necrosis of these slow-growing tumours.…”

“…The duodenum constitutes only the first 25 cm of the small bowel, yet about 40% of all small bowel carcinomas lie within this segment, while lymphoma and sarcoma are evenly distributed along the length of the bowel [7, 8]. It is therefore not surprising that only 15–28% of small bowel leiomyosarcomas arise from the duodenum and that barely 10% of all duodenal malignancies are of this type [4, 9, 10]. The distribution of these lesions within the duodenum in our series agrees with that reported in the literature, approximately half being reported in D2, one third in D3 and less than 10% in either D4 or D1 [1, 4, 11].…”

“…They tend to ulcerate into the small bowel causing either iron-deficiency anaemia or frank gastrointestinal haemorrhage. Weight loss, palpable mass and previous peptic ulcer are other common features that were present in our own patients [1, 4]. Endoscopy can highlight a submucosal lesion, with or without ulceration, which should be biopsied [13].…”

“…Sporadic case reports and occasional collected series make it difficult to determine the true diagnostic features and appropriate treatment of this rare tumour. Previous reviews [1, 4]have underlined the need for strict pathological criteria to confirm malignancy and for wide resection to obtain a cure. Surgical treatment and outcome are presented in 5 patients who may form the biggest series treated by a single surgeon.…”